Progression of Stargardt disease as determined by fundus autofluorescence over a 12-month period: ProgStar Report No. 11
JAMA Ophthalmology Oct 21, 2019
Strauss RW, Kong X, Ho A, et al. - In this multicenter prospective cohort study involving 259 candidates, researchers estimated the progression rate of atrophic lesions in the prospective Natural History of the Progression of Atrophy Secondary to Stargardt Disease study over a 12-month period. The study sample consisted of patients who were affected by Stargardt disease, aged 6 years and older at baseline, and harboring disease-causing variants of the ABCA4 gene at 9 centers in the United States, United Kingdom, and continental Europe. Data reported that the estimated progression of definitely decreased autofluorescence was 0.76 mm2 per year, and the total area of both questionable and definitely decreased autofluorescence was 0.64 mm2 per year. Results of this study suggested that autofluorescence imaging can be used as a surveillance instrument in Stargardt disease and certainly reduced autofluorescence and total area as result measures for interventional clinical trials aimed at slowing the development of the disease. Progress rate depended primarily on the size of the initial lesion.
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